Granulomatous cheilitis in a patient after SARS-CoV-2 infection treated with antibiotics: a case report.

Granulomatous cheilitis or Miescher's cheilitis is a rare granulomatous disorder defined by recurrent lip swelling or edema of other facial soft tissues. Histopathology shows non-caseous granulomas and multinucleated giant cells. The exact etiology is unknown, although genetic background, immunological irregularities, and systemic or infectious diseases contribute to the onset of disease. There are no treatment guidelines. The usual treatment options include systemic or intralesional corticosteroids, a spectrum of antibiotics, and immunosuppressants. A 63-year-old patient presenting with lip swelling and simultaneous swelling of other facial soft tissues was diagnosed with granulomatous cheilitis. The symptoms occurred 3 weeks after SARS-CoV-2 infection. Initial treatment with systemic corticosteroids and antihistamines was inadequate. Here we report successful treatment with a combination of doxycycline and metronidazole.

An uncommon case of lip swelling: Granulomatous cheilitis associated with Crohn's disease.

Unbeknownst to most otolaryngologists, there is quite a range of oral manifestations which commonly manifest in the context of inflammatory bowel disease. As providers who will encounter such patients in consultation it is beneficial to be aware of that association. Lip swelling (granulomatous cheilitis) is just one such presentation, which is often otherwise mistaken for angioneurotic edema and can lead to unwarranted testing and misdirected treatment. We present such a case to highlight the educational value of this patient encounter.

Parálisis facial periférica recidivante como manifestación inicial del síndrome de Melkersson-Rosenthal / Recurrent peripheral facial palsy as an initial manifestation of Melkersson-Rosenthal syndrome

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Melkersson-Rosenthal Syndrome Induced by COVID-19.

Melkersson-Rosenthal syndrome is a rare condition characterized by a triad of orofacial edema, facial paralysis, and fissured tongue. Histopathological examination of the disease has demonstrated areas of inflammation involving mast cells. Activated mast cells also play a part in the pathogenesis of COVID-19 infection, as they release cytokines in the lungs. We present a case of a female patient presenting with edema. We present a case of a female patient presenting with edema. Her examination revealed edema in the right lower lip, right facial paralysis, and fissured tongue. COVID-19 may be associated with which was not previously included in the etiology of the disease.

Oral lymphangiectasia and gastrointestinal Crohn disease.

Lip edema with non-caseating granulomas or lymphangiectasia pose a clinical and pathological challenge. These findings can be attributed to cheilitis granulomatosa (CG), Melkersson-Rosenthal syndrome (MRS), or Crohn disease (CD) depending on the appropriate clinical context. Lymphangiectasis, in particular, is a common pathological finding in CD due to lymphatic obstruction by granulomas and intralymphatic granulomas. Because oral symptoms can precede gastrointestinal symptoms of CD or be seen in patients with asymptomatic gastrointestinal disease, the identification of lymphangiectasia should raise the possibility of underlying CD. We present a case of a young woman with several years of lip swelling, with notable lymphangiectasia and subtle granulomas on pathological evaluation. The patient was diagnosed with MRS at an outside institution and treated with systemic steroids, without further systemic evaluation. We believe that early recognition of lymphangiectasia and consideration of CD early in the work-up are critical for early diagnosis and appropriate management. Neither clinical nor histopathological findings should be used in isolation to diagnose GC, MRS, or CD as there is significant debate as to the etiology and overlapping findings of these conditions. We highlight the importance of lymphangiectasia in diagnosing underlying CD in the appropriate clinical context.

Cheilitis granulomatosa and Crohn's disease : a case report.

In this case report we describe the evolution of Cheilitis granulomatosa (GC) in a young patient with Crohn's disease during treatment with anti-TNF-alfa therapy.

Melkersson-Rosenthal Syndrome: the possible role of comorbidities in the etiopathogenesis.

BACKGROUND: Melkersson-Rosenthal Syndrome (MRS) is a rare syndrome. Recently, possible association between MRS and psoriasis was reported. Our objective is to evaluate the presence of comorbidities in MRS with a focus on psoriasis-related morbidities. METHODS: We conducted a case-control study consisting of a series of 12 patients with MRS and two groups of age- and gender-matched controls: 30 patients with psoriasis vulgaris and 28 patients with acute contact dermatitis. A comparative analysis for the prevalence of comorbidities, with a focus on psoriasis-related morbidities, was done. RESULTS: Psoriasis-related morbidities including smoking, obesity, dyslipidemia, hypertension, and diabetes mellitus were recorded in 5 (42%) patients with MRS, compared to 15 (50%) patients with psoriasis and 2 (7%) patients with acute contact dermatitis. The prevalence of psoriasis-related morbidities did not differ significantly between the group of patients with MRS and the group of patients with psoriasis. On the other hand, the difference between the group of patients with MRS and the group of patients with contact dermatitis was statistically significant (P=0.01). CONCLUSIONS: The similar prevalence of psoriasis-related morbidities in MRS and in psoriasis may further support an association between MRS and psoriasis.

[Cheilitis granulomatosa revealing Crohn's disease]. / Macro-chéilite granulomatose révélant une maladie de Crohn.

Many disorders of the digestive tract cause cutaneous manifestations that may be an indication of an underlying condition; hence dermatologist plays a key role in recommending that the patient see a gastroenterologist. Conversely, gastroenterologist often sees patients with mucocutaneous lesions suggesting possible association with well-known digestive disorders. Chronic inflammatory bowel diseases are the typical example of this essential collaboration between the two specialities. We report a case of Crohn's disease revealed by cheilitis granulomatosa. This study involved a 30-year old female patient followed up for cheilitis granulomatosa in the Department of Dermatology. Somatic examination was unremarkable apart from dermatologic lesions (indurated white faciolabial edema and diffuse gingivitis). Biopsy of the labial lesion revealed perivascular epithelioid and giant-cell granulomas without caseous necrosis. Ileocolonoscopy was performed due to suspected inflammatory colitis. It showed gaping and ulcerated Bauhin valve with slightly erythematous ileal mucosa. Anatomo-pathologic study objectified chronic inflammatory bowel disease (CIBD) such as ileocolic Crohn's disease. Patient's evolution was marked by some episodes of rectal bleeding with anoperineal lesions 6 months later: a wide anterior anal fissure with aphthous ulceration 5 cm from the anal margin, complicated, a few weeks later, by the onset of perianal fistulized abscess requiring surgical drainage and seton installation. Hence the indication for thorough treatment of Crohn's disease after complete drying up of the abscess. Dermatologic lesions vary in patients with CIBD. In some cases, they occur in patients with IBD whereas, in other cases, they precede or accompany digestive manifestations, allowing diagnosis of sometimes clinically latent bowel disease.

[Macrocheilia revealing an acute promyelocytic leukemia]. / Macrochéilie révélant une leucémie aiguë promyélocytaire.

INTRODUCTION: Macrocheilia is a rare presentation for acute promyelocytic leukemia (APL). CASE REPORT: A 18 year-old man was referred to our department for an acute swollen lower lip, multiple swollen cervical lymph nodes and spontaneous gum bleeding. The diagnosis of APL was made and the treatment was rapidly started in an intensive care unit. DISCUSSION: APL is often found because of stomatologic symptoms but macrocheilia is rarely reported. APL is a vital emergency.

Case report of cheilitis granulomatosa and joint complaints as presentation of Crohn's disease.

Cheilitis granulomatosa is characterized by granulomatous lip swelling. We report a case of a 13-year-old girl who presented with orofacial swelling and arthralgia, who eventually was diagnosed with Crohn's disease, which was successfully treated with infliximab and azathioprine combination therapy. Recurrent or persistent orofacial swelling should prompt consideration of cheilitis granulomatosa, and further diagnostic evaluation to exclude the presence of Crohn's disease seems warranted.

Melkersson-Rosenthal syndrome as an early manifestation of mixed connective tissue disease.

PURPOSE OF REVIEW: We aim to illustrate the potential viability of MCTD as an underlying aetiology of Melkersson-Rosenthal syndrome. The case is probably the first description available in the literature of the Melkersson-Rosenthal as an early manifestation of mixed connective tissue disease. RECENT FINDINGS: The Melkersson-Rosenthal syndrome consists of a triad of recurrent lip and/or face swelling, fissured tongue, and intermittent facial palsy. Mixed connective tissue disease is a multisystemic disorder with overlapping features of systemic lupus erythematosus, scleroderma, and polymyositis, and is differentiated from them by a high titer of antibodies to ribonucleoprotein. The paper presents a case report of Melkersson-Rosenthal syndrome with an onset in childhood that derived from vasculitis that turned out to be an early manifestation of mixed connective tissue disease. We used MRI to evaluate patient's brain structure and Immunoblot Ena Profil 1 test to test serum autoantibodies level. The patient has a typical for Melkersson-Rosenthal syndrome triad of symptoms: bilateral facial nerve palsy, lingua plicata and facial oedema. Both TC and MRI of the head show no changes as well as laboratory tests except Anti-SS-A (Anti-Ro) and Anti-RNP autoantibody serum level that was highly positive. Neurological involvement of the MCTD usually includes, according to the frequency of the occurrence, trigeminal neuralgia, headaches, sensorineural hearing, cerebral haemorrhage, transverse myelitis, cauda equina syndrome, retinal vasculitis, progressive multifocal encephalopathy, and demyelinating neuropathy. For clinical practice it is important to remember that Melkersson-Rosenthal syndrome can also be the neurological manifestation of MCTD, especially when accompanied by other systemic symptoms.

Granulomatous cheilitis with intralymphatic histiocytosis possibly associated with calcium deposition caused by chronic inflammation owing to dental metals and periodontitis.

Granulomatous cheilitis (GC) is an uncommon disease and the pathogenesis of GC has not been clear. Here, we present two cases of GC associated with calcium deposition caused by chronic inflammation owing to dental metal allergy and periodontitis. Histopathologically, intralymphatic histiocytosis (IH) in addition to non-caseating granulomas was seen in both cases. The results of a patch test were positive for some dental metals. Qualitative analyses by energy-dispersive X-ray spectroscopy detected calcium from biopsy specimens. We considered that partial destruction of teeth by chronic inflammation induced reactive proliferation of histiocytes, resulting in IH. Our study suggests that GC and IH is a consequence of chronic inflammatory diseases caused by dental metals and periodontitis. Dental care is necessary for treatment of GC.

Buccal localization of Crohn's disease with long-term infliximab therapy: a case report.

INTRODUCTION: Cheilitis granulomatosa causes persistent idiopathic lip swelling and ulceration and it can sometimes be recognized as a unique or early manifestation of Crohn's disease. Spontaneous remission is rare and with the lack of controlled trials, different therapeutic approaches have been used. Some cases have been treated with an exclusion diet in the attempt to rule out diet allergens, while the most popular treatments include antibiotics such as tetracycline and clofazimine tranilast, benzocaine topical or intralesional steroids, and cheiloplasty, with different outcomes. CASE PRESENTATION: We describe the case of a 23-year-old Caucasian man, primarily diagnosed with cheilitis granulomatosa for a severe lower lip swelling, and then with Crohn's disease of the terminal ileum and anus. Treatment of Crohn's disease with an anti-tumor necrosis factor alpha agent (infliximab) successfully induced remission of both the gastrointestinal disease and the oral lesion. CONCLUSIONS: Our recommendation is that physicians should be able to recognize cheilitis granulomatosa as a possible marker of a more complex systemic disease and proceed first with an accurate physical examination, and further suggest investigations of the bowel. In cases of Crohn's disease, a therapy with biological agents can be successful.

Melkersson-Rosenthal syndrome: a review of seven patients.

Melkersson-Rosenthal syndrome (MRS) is a rare disorder consisting of a triad of persistent or recurrent orofacial edema, relapsing facial paralysis and fissured tongue. It is difficult to diagnose and treat. A retrospective review of our MRS patients was performed. The medical files, and treatment, radiologic and histopathologic records of these patients were reviewed. The study group consisted of seven male MRS patients. In four patients, the three classical manifestations were present simultaneously. Neuroimaging examinations found facial soft-tissue thickening and swelling in two patients. The indicators of the disease in our patients were as follows: two had family histories, two had elevated levels of protein in the cerebrospinal fluid, one had an increased immunoglobulin G level in the blood and one had reactive arthritis. All patients responded to systemic corticosteroid treatment. We discuss the genetic, infectious and immunologic factors in the etiology of MRS. Neuroimaging examinations were useful in determining the diagnosis and in determining whether the initial treatment of MRS should be with systemic corticosteroids.

Melkersson-Rosenthal syndrome in a patient with psoriatic arthritis receiving etanercept.

Melkersson-Rosenthal syndrome is a rare granulomatous neuro-mucocutaneous systemic disease that is characterized by relapsing peripheral facial paralysis, orofacial edema and fissured tongue. The disease etiology is still not well known, but it has been hypothesized that a possible role is played by various causal agents such as infectious diseases, genetic causes, allergic conditions, benign lymphogranulomatosis, various associations with other pathological conditions, particularly with immune-mediated diseases and food contact allergies. In this report we describe the case of a woman, 42 years old, with psoriatic arthritis who developed neurological episodes related to MRS after treatment with anti-TNF therapy. This finding further supports the hypothesis that TNF-alpha blockade, and particularly the use of the TNF-alpha receptor, could trigger the development of granulomatous lesions in predisposed patients. The case we report further sustains the importance for the clinician to take into account this potential adverse event in patients receiving anti-TNF-alpha therapies.

Melkersson-Rosenthal syndrome in a patient with tubercular panuveitis.

We report a rare presentation of Melkersson-Rosenthal syndrome in a patient with tubercular panuveitis. A 45-year-old male being treated with antitubercular therapy for tubercular panuveitis presented with unilateral, non-pitting right upper eyelid edema. Excision biopsy showed granulomatous inflammation involving the lymphatics. Immunohistochemistry confirmed the presence of histiocytes around the lymphatics.